The CamPaIGN study of Parkinson's disease: 10-year outlook in an incident population-based cohort

CH Williams-Gray, SL Mason, JR Evans… - Journal of Neurology …, 2013 - jnnp.bmj.com
Journal of Neurology, Neurosurgery & Psychiatry, 2013jnnp.bmj.com
Background Prognosis in Parkinson's disease (PD) remains poorly understood due to a lack
of unbiased data on the natural history of treated PD. The CamPaIGN study has been the
first to prospectively track disease evolution from diagnosis in an unselected population-
representative incident cohort. We now report the 10-year follow-up data, focusing on three
key irreversible milestones: postural instability (Hoehn and Yahr 3), dementia and death.
Methods The cohort was collected between December 2000 and 2002. Those meeting …
Background
Prognosis in Parkinson's disease (PD) remains poorly understood due to a lack of unbiased data on the natural history of treated PD. The CamPaIGN study has been the first to prospectively track disease evolution from diagnosis in an unselected population-representative incident cohort. We now report the 10-year follow-up data, focusing on three key irreversible milestones: postural instability (Hoehn and Yahr 3), dementia and death.
Methods
The cohort was collected between December 2000 and 2002. Those meeting diagnostic criteria (n=142) were followed-up until 1 January 2012. Clinical, neuropsychological and genetic testing were performed. Progression to key milestones was evaluated using Kaplan–Meier and Cox regression survival analyses.
Results
At 10 years, 55% had died, 68% had postural instability and 46% dementia. 23% had a good outcome at 10 years (surviving free of dementia/postural instability). Death rate was comparable with the UK population (standardised mortality ratio 1.29 (0.97–1.61)). Death certificates indicated PD was a substantial contributor in only 20%, with pneumonia being the commonest cause of death. Age, non-tremor-dominant motor phenotype and comorbidity predicted earlier postural instability. Baseline predictors of dementia were age, motor impairment, ‘posterior-cortical’ cognitive deficits and MAPT genotype.
Conclusions
(1) outlook in PD is heterogeneous, with most dying or developing dementia or postural instability by 10 years from diagnosis, but a quarter still doing well, with preserved mobility and intact cognition; (2) death is not directly related to PD in the majority; (3) baseline clinical and genetic variables are predictive of outcome and may be helpful in selecting patients for clinical trials.
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